Endoscopy plays a central role in diagnostic and therapeutic approaches to biliary disease in both benign and malignant conditions. A cholangioscope is an endoscopic instrument that allows for the direct exploration of the biliary tree. Over the years, technology has improved endoscopic image quality and allowed for the development of an operative procedure that can be performed during cholangioscopy. Different types of instruments are available in this context, and they can be used in different anatomical access points according to the most appropriate clinical indication. The direct visualization of biliary mucosa is essential in the presence of biliary strictures of unknown significance, allowing for the appropriate allocation of patients to surgery or conservative treatments. Cholangioscopy has demonstrated excellent performance in discriminating malignant conditions (such as colangiocarcinoma) from benign inflammatory strictures, and more recent advances (e.g., artificial intelligence and confocal laser endomicroscopy) could further increase its diagnostic accuracy. Cholangioscopy also plays a primary role in the treatment of benign conditions such as difficult bile stones (DBSs). In this case, it may not be possible to achieve complete biliary drainage using standard ERCP. Therapeutic cholangioscopy-guided lithotripsy allows for stone fragmentation and complete biliary drainage. Indeed, other complex clinical situations, such as patients with intra-hepatic lithiasis and patients with an altered anatomy, could benefit from the therapeutic role of cholangioscopy. The aim of the present review is to explore the most recent diagnostic and therapeutic advances in the roles of cholangioscopy in the management of biliary diseases.